Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies

Pietro Maggi, Martina Absinta, Matteo Grammatico, Luisa Vuolo, Giacomo Emmi, Giovanna Carlucci, Gregorio Spagni, Alessandro Barilaro, Anna Maria Repice, Lorenzo Emmi, Domenico Prisco, Vittorio Martinelli, Roberta Scotti, Niloufar Sadeghi, Gaetano Perrotta, Pascal Sati, Bernard Dachy, Daniel S. Reich, Massimo Filippi, Luca Massacesi

Research output: Contribution to journalArticle

Abstract

Objectives: In multiple sclerosis (MS), magnetic resonance imaging (MRI) is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the “central vein sign”) improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as central nervous system (CNS) inflammatory vasculopathies, is lacking. In a multicenter study, we assessed the frequency of perivenular lesions in MS versus systemic autoimmune diseases with CNS involvement and primary angiitis of the CNS (PACNS). Methods: In 31 patients with inflammatory CNS vasculopathies and 52 with relapsing–remitting MS, 3-dimensional T2*-weighted and T2–fluid-attenuated inversion recovery images were obtained during a single MRI acquisition after gadolinium injection. For each lesion, the central vein sign was evaluated according to consensus guidelines. For each patient, lesion count, volume, and brain location, as well as fulfillment of dissemination in space MRI criteria, were assessed. Results: MS showed higher frequency of perivenular lesions (median = 88%) than did inflammatory CNS vasculopathies (14%), without overlap between groups or differences between 3T and 1.5T MRI. Among inflammatory vasculopathies, Behçet disease showed the highest median frequency of perivenular lesions (34%), followed by PACNS (14%), antiphospholipid syndromes (12%), Sjögren syndrome (11%), and systemic lupus erythematosus (0%). When a threshold of 50% perivenular lesions was applied, central vein sign discriminated MS from inflammatory vasculopathies with a diagnostic accuracy of 100%. Interpretation: The central vein sign differentiates inflammatory CNS vasculopathies from MS at standard clinical magnetic field strengths. Ann Neurol 2018;83:283–294.

Original languageEnglish (US)
Pages (from-to)283-294
Number of pages12
JournalAnnals of neurology
Volume83
Issue number2
DOIs
StatePublished - Feb 1 2018
Externally publishedYes

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Multiple Sclerosis
Veins
Central Nervous System
Magnetic Resonance Imaging
Autoimmune Diseases
Central Nervous System Vasculitis
Antiphospholipid Syndrome
Gadolinium
Brain
Magnetic Fields
Vasculitis
Systemic Lupus Erythematosus
Multicenter Studies
Biomarkers
Guidelines
Injections

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies. / Maggi, Pietro; Absinta, Martina; Grammatico, Matteo; Vuolo, Luisa; Emmi, Giacomo; Carlucci, Giovanna; Spagni, Gregorio; Barilaro, Alessandro; Repice, Anna Maria; Emmi, Lorenzo; Prisco, Domenico; Martinelli, Vittorio; Scotti, Roberta; Sadeghi, Niloufar; Perrotta, Gaetano; Sati, Pascal; Dachy, Bernard; Reich, Daniel S.; Filippi, Massimo; Massacesi, Luca.

In: Annals of neurology, Vol. 83, No. 2, 01.02.2018, p. 283-294.

Research output: Contribution to journalArticle

Maggi, P, Absinta, M, Grammatico, M, Vuolo, L, Emmi, G, Carlucci, G, Spagni, G, Barilaro, A, Repice, AM, Emmi, L, Prisco, D, Martinelli, V, Scotti, R, Sadeghi, N, Perrotta, G, Sati, P, Dachy, B, Reich, DS, Filippi, M & Massacesi, L 2018, 'Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies', Annals of neurology, vol. 83, no. 2, pp. 283-294. https://doi.org/10.1002/ana.25146
Maggi, Pietro ; Absinta, Martina ; Grammatico, Matteo ; Vuolo, Luisa ; Emmi, Giacomo ; Carlucci, Giovanna ; Spagni, Gregorio ; Barilaro, Alessandro ; Repice, Anna Maria ; Emmi, Lorenzo ; Prisco, Domenico ; Martinelli, Vittorio ; Scotti, Roberta ; Sadeghi, Niloufar ; Perrotta, Gaetano ; Sati, Pascal ; Dachy, Bernard ; Reich, Daniel S. ; Filippi, Massimo ; Massacesi, Luca. / Central vein sign differentiates Multiple Sclerosis from central nervous system inflammatory vasculopathies. In: Annals of neurology. 2018 ; Vol. 83, No. 2. pp. 283-294.
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abstract = "Objectives: In multiple sclerosis (MS), magnetic resonance imaging (MRI) is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the “central vein sign”) improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as central nervous system (CNS) inflammatory vasculopathies, is lacking. In a multicenter study, we assessed the frequency of perivenular lesions in MS versus systemic autoimmune diseases with CNS involvement and primary angiitis of the CNS (PACNS). Methods: In 31 patients with inflammatory CNS vasculopathies and 52 with relapsing–remitting MS, 3-dimensional T2*-weighted and T2–fluid-attenuated inversion recovery images were obtained during a single MRI acquisition after gadolinium injection. For each lesion, the central vein sign was evaluated according to consensus guidelines. For each patient, lesion count, volume, and brain location, as well as fulfillment of dissemination in space MRI criteria, were assessed. Results: MS showed higher frequency of perivenular lesions (median = 88{\%}) than did inflammatory CNS vasculopathies (14{\%}), without overlap between groups or differences between 3T and 1.5T MRI. Among inflammatory vasculopathies, Beh{\cc}et disease showed the highest median frequency of perivenular lesions (34{\%}), followed by PACNS (14{\%}), antiphospholipid syndromes (12{\%}), Sj{\"o}gren syndrome (11{\%}), and systemic lupus erythematosus (0{\%}). When a threshold of 50{\%} perivenular lesions was applied, central vein sign discriminated MS from inflammatory vasculopathies with a diagnostic accuracy of 100{\%}. Interpretation: The central vein sign differentiates inflammatory CNS vasculopathies from MS at standard clinical magnetic field strengths. Ann Neurol 2018;83:283–294.",
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AU - Absinta, Martina

AU - Grammatico, Matteo

AU - Vuolo, Luisa

AU - Emmi, Giacomo

AU - Carlucci, Giovanna

AU - Spagni, Gregorio

AU - Barilaro, Alessandro

AU - Repice, Anna Maria

AU - Emmi, Lorenzo

AU - Prisco, Domenico

AU - Martinelli, Vittorio

AU - Scotti, Roberta

AU - Sadeghi, Niloufar

AU - Perrotta, Gaetano

AU - Sati, Pascal

AU - Dachy, Bernard

AU - Reich, Daniel S.

AU - Filippi, Massimo

AU - Massacesi, Luca

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N2 - Objectives: In multiple sclerosis (MS), magnetic resonance imaging (MRI) is a sensitive tool for detecting white matter lesions, but its diagnostic specificity is still suboptimal; ambiguous cases are frequent in clinical practice. Detection of perivenular lesions in the brain (the “central vein sign”) improves the pathological specificity of MS diagnosis, but comprehensive evaluation of this MRI biomarker in MS-mimicking inflammatory and/or autoimmune diseases, such as central nervous system (CNS) inflammatory vasculopathies, is lacking. In a multicenter study, we assessed the frequency of perivenular lesions in MS versus systemic autoimmune diseases with CNS involvement and primary angiitis of the CNS (PACNS). Methods: In 31 patients with inflammatory CNS vasculopathies and 52 with relapsing–remitting MS, 3-dimensional T2*-weighted and T2–fluid-attenuated inversion recovery images were obtained during a single MRI acquisition after gadolinium injection. For each lesion, the central vein sign was evaluated according to consensus guidelines. For each patient, lesion count, volume, and brain location, as well as fulfillment of dissemination in space MRI criteria, were assessed. Results: MS showed higher frequency of perivenular lesions (median = 88%) than did inflammatory CNS vasculopathies (14%), without overlap between groups or differences between 3T and 1.5T MRI. Among inflammatory vasculopathies, Behçet disease showed the highest median frequency of perivenular lesions (34%), followed by PACNS (14%), antiphospholipid syndromes (12%), Sjögren syndrome (11%), and systemic lupus erythematosus (0%). When a threshold of 50% perivenular lesions was applied, central vein sign discriminated MS from inflammatory vasculopathies with a diagnostic accuracy of 100%. Interpretation: The central vein sign differentiates inflammatory CNS vasculopathies from MS at standard clinical magnetic field strengths. Ann Neurol 2018;83:283–294.

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